Histological origin of pseudomyxoma peritonei in Chinese women: clinicopathology and immunohistochemistry

Abstract

Aim: To investigate the histological origin of pseudomyxoma peritonei (PMP) in Chinese women.

Methods: The clinical and pathological data were reviewed for 35 women with PMP, and specimens of the peritoneal, appendiceal and ovarian lesions of each patient were examined using the PV-6000 immunohistochemistry method. Antibodies included cytokeratin (CK)7, CK20, mucin (MUC)-1, MUC-2, carbohydrate antigen (CA)-125, estrogen receptor (ER), and progesterone receptor (PR).

Results: Abundant colloidal mucinous tumors were observed in the peritoneum in all 35 cases. Thirty-one patients had a history of appendectomy, 28 of whom had mucinous lesions. There was one patient with appendicitis, one whose appendix showed no apparent pathological changes, and one with unknown surgical pathology. Ovarian mucinous tumors were found in 24 patients. The tumors were bilateral in 13 patients, on the right-side in nine, and on the left side in two. Twenty patients had combined appendiceal and ovarian lesions; 16 of whom had undergone initial surgery for appendiceal lesions. Four patients had undergone initial surgery for ovarian lesions, and relapse occurred in these patients at 1, 11, 32 and 85 mo after initial surgery. Appendiceal mucinous tumors were found in each of these four patients. Thirty-three of the 35 patients showed peritoneal lesions that were positive for CK20 and MUC-2, but negative for CK7, MUC-1, CA125, ER and PR. The expression patterns in the appendix and the ovary were similar to those of the peritoneal lesions. In one of the remaining two cases, CK20, CK7 and MUC-2 were positive, and MUC-1, CA125, ER and PR were negative. The ovaries were not resected. The appendix of one patient was removed at another hospital, and no specimen was evaluated. In the other case, the appendix appeared to be normal during surgery, and was not resected. Peritoneal and ovarian lesions were negative for CK20, MUC-2, CK7, MUC-1, CA125, ER and PR.

Conclusion: Most PMP originated from the appendix. Among women with PMP, the ovarian tumors were implanted rather than primary. For patients with PMP, appendectomy should be performed routinely. The ovaries, especially the right ovaries should be explored.

Keywords: Appendix; Immunohistochemistry; Ovary; Peritoneum; Pseudomyxoma peritonei; Tumor origin.

Figure 1

Upon sectioning, the peritoneal lesions were full of a jelly-like mucous substance.

Figure 2

Microscopically, some mucous glandular structures were floating in a large number of mucous lakes (HE stain, × 200).

Figure 3

Mucinous tumors were observed in the appendix (A) (HE stain, ×100) and ovary (B) (HE stain, × 100).

Figure 4

Mucin-2 was positive in peritoneal (A) (× 200), appendiceal (B) (× 100) and ovarian (C) (× 100) lesions.

Figure 5

Survival of 35 women with pseudomyxoma peritonei.